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Pleuroparenchymal fibroelastosis (PPFE) is a rare progressive interstitial lung disease presenting with predominantly upper-lobe pleural thickening and pulmonary fibrosis. While most cases are idiopathic, it is rarely seen as a complication of infection or following chemotherapy, radiation therapy, and bone marrow transplant. We describe a case of early onset and rapidly progressive PPFE as a complication of cyclophosphamide chemotherapy.

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